P-008: PREVALENCE AND PREDICTORS OF PULMONARY HYPERTENSION IN PAEDIATRIC SICKLE CELL DISEASE
Authors: LAKHERA T., MERCHANT S.
Journal: HemaSphere (2022)

Purpose: Pulmonary Hypertension(PHTN) is a well-described, serious, and potentially life-threatening complication of hemolytic anaemia, including Sickle Cell Disease(SCD) and is associated with a significant increase in morbidity and mortality rate.In the paediatric population,however,death and morbidity is much less common and has not been well defined. In the past, a significant association of Pulmonary Hypertension with Tricuspid jet velocity and laboratory markers of hemolysis has been found. However there is paucity of data in this subject. Hence,it is important to study the prevalence of pulmonary hypertension and to identify the variables predictive of PHTN in this population that will benefit patients in early identification and appropriate intervention. Materials and methods: A cross sectional study was conducted considering a total of 91 patients between 5-18 years, with SCD (homozygous) of either sex, diagnosed using HPLC attending outpatient clinic in their steady state for routine clinical care at a tertiary care hospital. Patients with history of acute illness or any SCD related complication within 4 weeks were excluded from the study.At the time of enrolment, detailed history, demographic data, age at diagnosis, details of blood transfusion and Hydroxyurea were taken.Detailed clinical examination of study subjects was recorded in structured data sheet.Investigations done were complete hemogram, reticulocyte count,LDH,liver function tests,renal function tests,pulse oximetry, chest radiograph, electrocardiogram and 2D echocardiogram.Pulmonary hypertension was defined as a peak TRV of at least 2.5 m/second equating to a pulmonary artery pressure of at least 30 mm Hg. All the data was validated and analyzed in statistical software STATA, version 10.1,2011. Results: Total 91 patients (51 males, 40 females) between the age group of 5-18 years were enrolled in the study with mean age of 10.68 ± 4.03 years.Overall prevalence of pulmonary hypertension (PHT) was 11%. Mild PHT (TR Jet velocity 2.5 to 2.9m/second) was found in 8(8.79%) patients and 2 (2.20%) patients had moderate to severe PHT (TR jet velocity >3 m/second).The mean age of the patient with pulmonary hypertension was found out to be 14.7+-2.26 and there was significant association between pulmonary hypertension and increasing age(p value=0.0011).High reticulocyte count(p-value<0.0001),increased serum bilirubin(p value<0.0075),raised serum lactate dehydrogenase (LDH) (p value<0.0004), high total leucocyte count(p-value<0.0001), lower platelet count (p-value 0.0018),low hemoglobin (p=0.0061), more frequent packed red cell transfusions (p value-0.0001) were significantly associated with elevated pulmonary artery pressures. On MLR analysis,only Age,Reticulocyte count and BT rate were found to be significant.In the study,there was no association of Hydroxyurea therapy with lesser prevalence of PHTN.Also,there was no significant association of gender or oxygen saturation between patients with and without PHTN. Conclusion: High pulmonary artery pressures do occur in children with SCD. This emphasizes the need for early screening of Pulmonary Hypertension by echocardiography for early detection and intervention in paediatric patients with SCD The authors do not declare any conflict of interest